From the Guidelines
Angiosarcoma is a rare and aggressive cancer that develops in the inner lining of blood vessels and lymph vessels, and its treatment typically involves a combination of surgery, radiation therapy, and chemotherapy, with paclitaxel being a potential first or second-line treatment option 1.
Definition and Characteristics
Angiosarcoma can occur anywhere in the body but most commonly affects the skin, breast, liver, and spleen. It is a highly aggressive cancer with a high risk of local relapse and metastatic spread, especially in cases like angiosarcoma of the breast 1.
Treatment Approaches
For localized angiosarcoma:
- Surgery: Wide excision of the tumor with clear margins is the primary treatment.
- Radiation therapy: Often used before or after surgery to reduce tumor size or prevent recurrence.
- Chemotherapy: May be used in addition to surgery and radiation, especially for high-risk cases.
For metastatic angiosarcoma:
- Chemotherapy: The main treatment, often using drugs like paclitaxel, doxorubicin, or gemcitabine.
- Targeted therapy: Drugs like pazopanib or sorafenib may be used to block tumor growth.
Chemotherapy Regimens
- Paclitaxel: May be used as first or second-line treatment of angiosarcomas 1.
- Other options like liposomal doxorubicin, oral cyclophosphamide, and prednisolone may also be considered based on the patient's condition and previous treatments 1.
Importance of Early Detection and Treatment
Due to the aggressive nature of angiosarcoma, early detection and prompt treatment are crucial. Regular follow-up appointments and imaging studies are necessary to monitor for recurrence. The prognosis is generally poor, with 5-year survival rates ranging from 20-35%, emphasizing the need for ongoing research into more effective treatments 1.
From the Research
Definition and Characteristics of Angiosarcoma
- Angiosarcoma is a rare and aggressive malignancy with a poor prognosis 2
- It is a type of soft tissue sarcoma that originates from endothelial cells 3
- Angiosarcoma typically occurs in the skin of the head and neck in the elderly population 4
- It can occur anywhere in the body, but is most commonly found in the head, neck, and scalp 3
Risk Factors and Causes
- Previous radiation treatment is a risk factor for developing angiosarcoma 2, 3
- Chronic lymphedema is also a risk factor for angiosarcoma 3, 5
- Certain genetic changes are suspected to contribute to the development of angiosarcoma 3
- Environmental carcinogens and certain familial syndromes may also play a role in the development of angiosarcoma 5
Diagnosis and Treatment
- Diagnosis of angiosarcoma requires pathological and immunohistochemical confirmation 5
- Ultrasound, CT, and MR are diagnostic tools that can be used to aid in diagnosis 5
- Treatment options for angiosarcoma include surgery, radiotherapy, and chemotherapy 2, 4, 6, 5
- Targeted medicines and immunotherapy have been studied as promising treatments for angiosarcoma 3, 5
- A multidisciplinary approach, including surgery, radiotherapy, systemic chemotherapy, or immunotherapy, is typically the most effective way to achieve favorable outcomes 3